Absent T-cell immunity resulting in life-threatening infections provides a clear rationale for hematopoetic stem cell transplantation (HSCT) in patients with severe combined immunodeficiency (SCID). Combined immunodeficiencies (CIDs) and "atypical" SCID show reduced, not absent T-cell immunity. If associated with infections or autoimmunity, they represent profound combined immunodeficiency (P-CID), for which outcome data are insufficient for unambiguous early transplant decisions.

Speckmann, C., Doerken, S., Aiuti, A., Albert, M.h., Al-Herz, W., Allende, L.m., et al. (2017). A prospective study on the natural history of patients with profound combined immunodeficiency: An interim analysis. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, 139(4) [10.1016/j.jaci.2016.07.040].

A prospective study on the natural history of patients with profound combined immunodeficiency: An interim analysis

Aiuti, Alessandro;Cancrini, Caterina;Di Cesare, Silvia;Finocchi, Andrea;
2017-04-01

Abstract

Absent T-cell immunity resulting in life-threatening infections provides a clear rationale for hematopoetic stem cell transplantation (HSCT) in patients with severe combined immunodeficiency (SCID). Combined immunodeficiencies (CIDs) and "atypical" SCID show reduced, not absent T-cell immunity. If associated with infections or autoimmunity, they represent profound combined immunodeficiency (P-CID), for which outcome data are insufficient for unambiguous early transplant decisions.
apr-2017
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA
English
Con Impact Factor ISI
T-cell deficiency; combined immunodeficiency; hematopoietic stem cell transplantation; natural history; Adolescent; Child; Child, Preschool; Female; Humans; Infant; Male; Prospective Studies; Research Design; Severe Combined Immunodeficiency; Hematopoietic Stem Cell Transplantation
Speckmann, C., Doerken, S., Aiuti, A., Albert, M.h., Al-Herz, W., Allende, L.m., et al. (2017). A prospective study on the natural history of patients with profound combined immunodeficiency: An interim analysis. THE JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY, 139(4) [10.1016/j.jaci.2016.07.040].
Speckmann, C; Doerken, S; Aiuti, A; Albert, Mh; Al-Herz, W; Allende, Lm; Scarselli, A; Avcin, T; Perez-Becker, R; Cancrini, C; Cant, A; Di Cesare, S; Finocchi, A; Fischer, A; Gaspar, Hb; Ghosh, S; Gennery, A; Gilmour, K; González-Granado, Li; Martinez-Gallo, M; Hambleton, S; Hauck, F; Hoenig, M; Moshous, D; Neven, B; Niehues, T; Notarangelo, L; Picard, C; Rieber, N; Schulz, A; Schwarz, K; Seidel, Mg; Soler-Palacin, P; Stepensky, P; Strahm, B; Vraetz, T; Warnatz, K; Winterhalter, C; Worth, A; Fuchs, S; Uhlmann, A; Ehl, S
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/310237
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